Craniofacial surgery for craniosynostosis: challenges in diagnosis, management and long-term outcome

Craniofacial surgery for craniosynostosis is one of the most challenging reconstructive procedures. Restoration of particular functional and anatomic requirements is important for development from infancy to adulthood. The purpose of this study is to present the authors' experience of craniofacial surgery for management of patients with craniosynostosis in Srinagarind Hospital, Khon Kaen, Thailand, addressing the challenges of diagnosis, management and outcomes, which may be adapted in other developing countries.

This paper presents the cranial and associated deformities, diagnosis, radiologic findings, preoperative evaluation, craniofacial and maxillary surgeries and outcome(s) of patients with craniosynostosis. The care team, made up of neurosurgeons, plastic surgeons, radiologists, ophthalmologists and pediatricians, established the Tawanchai Center's protocol for craniosynostosis, to manage the timing of craniofacial procedures from infancy to adulthood.

The physical examination and radiologic findings of three patients, two with sagittal synostosis and one with plagiocephaly are reported. The clinical, craniofacial and maxillofacial surgeries and long-term outcomes of another three patients were studied one with Apert syndrome and two with Crouzon syndrome. All the latter three patients were lost to follow-up after the initial post-surgical visit. At that time, there were appropriate surgical results vis-a-vis appearance and satisfaction from the perspective of the two patients with Crouzon syndrome and their families. One of the patients with Crouzon syndrome received normal education supported by a successful family, while the other was still continuing her studies at school with good progress. The patient with Apert syndrome continued to live with his parents. Additional reconstructive surgery is recommended for all three patients. Economic problems and lack of adequate information were the main reasons for their discontinuing follow-up appointments.

Systematic physical examination and radiologic assessments by the craniofacial team are critically important for diagnosis, evaluation, planning of management and outcome assessment of the patients with craniosynostosis. In Thailand and other developing countries, the challenges in management of these patients are the development of standard craniofacial surgery, craniofacial team management and well-coordinated care, planned surgeries and outcome assessments from infancy to adolescence. A supportive government health system and establishment of a craniofacial center and foundation is needed in order to support and provide proper care for these groups of patients.